A Clearer View of Upper Motor Neuron Dysfunction in Amyotrophic Lateral Sclerosis

Elliott, Jeffrey L.

doi:10.1001/archneur.55.7.910

Cited by 3 publications

(4 citation statements)

References 13 publications

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“…Notably, our study also demonstrated decreased FA in the CC of patients with ALS, which was driven by changes in the transcallosal Figure 2. Mean FA and RD values for ALS patients plotted against the control group's mean FA and RD values for each CC area (1)(2)(3)(4)(5). For both DTI measures, there was a significant effect between-group membership and location in the CC, and post hoc t-tests comparing groups by individual CC area that resulted in significant differences with Bonferroni correction are indicated with an asterisk (*,P < 0.01).…”

Section: Discussionmentioning

confidence: 99%

“…It has been stated that there is a need for an objective early marker for UMN impairment in ALS. Using the current clinically guided methods of diagnosis, disease‐related neurodegeneration outside the traditional motor network of the CST is not easily observed . A notable exception is the mirror movement, an involuntary movement that mirrors that of a contralateral limb.…”

Section: Discussionmentioning

confidence: 99%

“…Unfortunately, early diagnosis of ALS has proven elusive as clinical confirmation can only occur after both upper (UMN) and lower motor neuron (LMN) involvement are detected by neurologic examination, which often occurs at a time point of advanced pathology . While noninvasive neurophysiologic techniques exist to detect subclinical LMN dysfunction, there is no sensitive tool that can similarly distinguish UMN dysfunction to aid in early diagnosis . For these reasons, much attention has turned toward neuroimaging as a means of identifying UMN involvement .…”

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confidence: 99%

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Diffusion tensor MRI of the corpus callosum in amyotrophic lateral sclerosis

Chapman

Jelsone-Swain

Johnson

et al. 2013

Magnetic Resonance Imaging

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Purpose To determine if decline in corpus callosum (CC) white matter integrity in patients with amyotrophic lateral sclerosis (ALS) is localized to motor-related areas. Materials and Methods Twenty-one ALS patients and 21 controls participated. Diffusion tensor images (DTI) were acquired using 3 Tesla (T) MRI. Tract-based spatial statistics were used to examine whole-brain white matter damage. A segmentation schema was used to define CC volumes-of-interest (VOI). Fractional anisotropy (FA) and radial- and axial-diffusivity (RD, AD) were extracted from VOIs and compared between groups. DTI measurements in motor-related Area III were tested for correlation with symptoms and disease duration. Results Extracted FA values from CC VOIs were reduced in ALS patients (P≤0.0001), particularly in Areas II and III (P≤0.01). Reduced FA in Area III correlated with disease symptomology (P≤0.05) and duration (P≤0.02). Between-group whole-brain comparisons (P≤0.05, corrected) showed reduced FA and increased RD throughout white matter regions including the CC, corona radiata, and internal capsule. AD was increased in the left corona radiata and internal and external capsules. Conclusion FA in motor-related regions of the CC is more affected than other CC areas in ALS patients. Microstructural pathology of transcallosal fiber tracts may represent a future component of an imaging biomarker for ALS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Diffusion tensor MRI of the corpus callosum in amyotrophic lateral sclerosis

Chapman

Jelsone-Swain

Johnson

et al. 2013

Magnetic Resonance Imaging

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“…An alternative explanation could be that ALS patients have a lower concentration of NAA in their neurons due to metabolic changes. Although NAA in ALS as first assumed is not correlated with the total perikaryon volume or cell number, mean NAA might still be effective in monitoring disease progress and evaluating treatment, and may possibly be helpful in distinguishing between pure LMN diseases and ALS in patients with only LMN symptoms (Elliott, 1998) .…”

Section: Discussionmentioning

confidence: 99%

The size distribution of neurons in the motor cortex in amyotrophic lateral sclerosis

Toft¹,

Gredal

Pakkenberg³

2005

Journal of Anatomy

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The motor cortex of eight patients with amyotrophic lateral sclerosis (ALS) and nine control subjects was used in the study. Recent stereological tools, the disector and the rotator method, were applied to the motor cortex of patients with ALS and control subjects to obtain estimates of mean perikaryon volume, mean neuronal nuclear volume, total perikaryon volume and total neuronal nuclear volume. No significant differences were found in any of the estimates. In vivo proton magnetic resonance spectroscopy studies show a decrease in the concentration of neuronal markers. We expected to find changes in perikaryon and/or nuclei neuronal volume because the total neuron number is unchanged in ALS compared with control subjects. However, this was not the case; our results suggest that metabolic changes take place in the motor cortex of ALS patients without these concomitant anatomical changes.

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